PEX13 Antibodies, cDNA Clones Research Reagents

PEX13 (Peroxisomal Biogenesis Factor 13, also known as ZWS; NALD; PBD11A; PBD11B), located on 2p15, is a Protein Coding gene. This gene encodes a peroxisomal membrane protein that binds the type 1 peroxisomal targeting signal receptor via an SH3 domain located in the cytoplasm. The gene produces a 44130 Da protein composed of 403 amino acids. PEX13 is an integral membrane protein on the peroxisome that regulates peroxisomal matrix protein import during peroxisome biogenesis. Diseases such as Peroxisome Biogenesis Disorder 11B and Peroxisome Biogenesis Disorder 11A are associated with PEX13.

PEX13 Antibody (1)

    PEX13 cDNA Clone (28)

    BC067090

    クローニングベクター cDNA 製品

    In lentiviral vector

    NM_023651.4

    クローニングベクター cDNA 製品

    In lentiviral vector

    PEX13 qPCR Primer (1)

    PEX13 の背景知識

    Pex13 encodes an SH3-containing peroxisomal membrane protein required for the import of proteins into peroxisomes. PEX13 is an integral membrane protein on the peroxisome that regulates peroxisomal matrix protein import during peroxisome biogenesis. PEX13 spans approximately 11 kb on chromosome 2 and contains four exons, one more than previously thought. The corrected PEX13 cDNA is predicted to encode a protein product with a molecular mass of 44,312 Da. Mutations in PEX13 and other peroxin proteins are associated with Zellweger syndrome spectrum (ZSS) disorders, a subtype of peroxisome biogenesis disorder characterized by prominent neurological, hepatic, and renal abnormalities leading to neonatal death. In humans, mutations in PEX13 can disrupt peroxisome biogenesis and lead to peroxisomal metabolic dysfunction and neurodegenerative disease.

    PEX13 の参考文献

    • Maxwell M, et al. (2003) Pex13 inactivation in the mouse disrupts peroxisome biogenesis and leads to a zellweger syndrome phenotype. Mol Cell Biol 23 (16): 5947-5957.
    • Lee MY, et al. (2017) Peroxisomal protein pex13 functions in selective autophagy. EMBO Rep 18 (1): 48-60.
    • Björkman J, et al. (2002) Pex13, the mouse ortholog of the human peroxisome biogenesis disorder pex13 gene: Gene structure, tissue expression, and localization of the protein to peroxisomes. Genomics 79 (2): 162-168.
    • Björkman J, et al. (1998) Genomic structure of pex13, a candidate peroxisome biogenesis disorder gene. Genomics 54 (3): 521-528.

    Note: Flag® is a registered trademark of Sigma Aldrich Biotechnology LP. It is used here for informational purposes only.